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Original Research: PULMONARY HYPERTENSION |

Pulmonary Arterial Hypertension: Baseline Characteristics From the REVEAL Registry

David B. Badesch, MD, FCCP; Gary E. Raskob, PhD; C. Greg Elliott, MD, FCCP; Abby M. Krichman, BS, RRT; Harrison W. Farber, MD, FCCP; Adaani E. Frost, MD, FCCP; Robyn J. Barst, MD, FCCP; Raymond L. Benza, MD; Theodore G. Liou, MD, FCCP; Michelle Turner, MS; Scott Giles, BA; Kathy Feldkircher, PhD; Dave P. Miller, MS; Michael D. McGoon, MD, FCCP
Author and Funding Information

From the Divisions of Pulmonary Sciences and Critical Care Medicine, and Cardiology, University of Colorado Denver (Dr Badesch), Denver, CO; the College of Public Health, University of Oklahoma Health Sciences Center (Dr Raskob), Oklahoma City, OK; the Department of Medicine, Intermountain Medical Center and the University of Utah (Dr Elliott), Salt Lake City, UT; the Pulmonary Vascular Disease Center, Duke University Medical Center (Ms Krichman), Durham, NC; the Pulmonary Hypertension Center, Boston University Medical Center (Dr Farber), Boston, MA; the Department of Medicine, Baylor College of Medicine (Dr Frost), Houston, TX; College of Physicians & Surgeons, Columbia University (Dr Barst), New York, NY; Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital (Dr Benza), Pittsburgh, PA; the Pulmonary Department, University of Utah (Dr Liou), Salt Lake City, UT; ICON Clinical Research (Ms Turner and Mr Miller), San Francisco, CA; Actelion Pharmaceuticals US, Inc. (Mr Giles and Dr Feldkircher), South San Francisco, CA; and the Division of Cardiovascular Diseases, Mayo Clinic (Dr McGoon), Rochester, MN.

Correspondence to: David B. Badesch, MD, FCCP, Professor of Medicine, Divisions of Pulmonary Sciences and Critical Care Medicine, and Cardiology, Clinical Director, Pulmonary Hypertension Center, University of Colorado Denver, Leprino Building, Room 536 or Box 957, 12401 E. 17th Ave, Aurora, CO 80045; e-mail: david.badesch@ucdenver.edu


Funding/Support: The REVEAL Registry is sponsored by Actelion Pharmaceuticals. Editorial support for the preparation of this manuscript was funded by Actelion Pharmaceuticals and provided by Jennifer M. Kulak, PhD, and Carol A. Lewis, PhD, from Wolters Kluwer.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2010 American College of Chest Physicians


Chest. 2010;137(2):376-387. doi:10.1378/chest.09-1140
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Background:  The Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL Registry) was established to provide updated characteristics of patients with pulmonary arterial hypertension (PAH) and to improve diagnosis, treatment, and management.

Methods:  Fifty-four US centers enrolled consecutively screened patients with World Health Organization group I PAH who met expanded hemodynamic criteria of mean pulmonary arterial pressure (PAP) > 25 mm Hg at rest (30 mm Hg with exercise), pulmonary capillary wedge pressure (PCWP) ≤ 18 mm Hg, and pulmonary vascular resistance ≥ 240 dynes · s · cm−5. Patients meeting the traditional hemodynamic definition (PCWP ≤ 15 mm Hg) were compared with those with a PCWP of 16 to 18 mm Hg.

Results:  Between March 2006 and September 2007, 2,967 patients enrolled. Among 2,525 adults meeting traditional hemodynamic criteria, the mean age was 53 ± 14 years, and 2,007 (79.5%) were women. The mean duration between symptom onset and diagnostic catheterization was 2.8 years, and 1,008 (41.3%) patients were treated with more than one pulmonary vascular-targeted medication. Compared with patients meeting the traditional hemodynamic definition of PAH, patients with a PCWP of 16 to 18 mm Hg were older, more obese, had a lower 6-min walk distance, and had a higher incidence of systemic hypertension, sleep apnea, renal insufficiency, and diabetes.

Conclusions:  Patients in the REVEAL Registry are older and more often female than in previous descriptions. Delays between symptom onset and diagnostic catheterization persist. Many treatment regimens are fundamentally empirical, and data will be required to determine outcomes, improve risk stratification, and develop and validate more precise prognostic tools. Patients with PCWP of 16 to 18 mm Hg differ in a number of important respects from those meeting the traditional hemodynamic definition of PAH.

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